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Pulmonary arterial hypertension associated with congenital heart disease

Usha Krishnan1, Erika B Rosenzweig

  • 1Columbia University Medical Center, Pulmonary Hypertension Center, Division of Pediatric Cardiology, 3959 Broadway, CH-2N, New York, NY 10032, USA.

Clinics in Chest Medicine
|November 26, 2013
PubMed

Insights

Adults with congenital heart disease (CHD) increasingly face pulmonary arterial hypertension (PAH) associated with CHD (APAH-CHD). This review covers APAH-CHD forms, hemodynamic interpretation, patient classification, and current treatments.

Area of Science:

  • Cardiology
  • Pulmonology
  • Adult Congenital Heart Disease

Background:

  • Growing number of adults with congenital heart disease (CHD) surviving into adulthood.
  • Pulmonary arterial hypertension (PAH) is a significant complication in this population, termed APAH-CHD.
  • Management challenges arise from complex underlying conditions and late repairs.

Purpose of the Study:

  • To review common forms of pulmonary arterial hypertension associated with congenital heart disease (APAH-CHD).
  • To guide the interpretation of hemodynamic data in APAH-CHD patients.
  • To classify APAH-CHD patients into clinically relevant subgroups for tailored management.

Main Methods:

  • Literature review focusing on APAH-CHD.
  • Analysis of hemodynamic parameters for classification.
  • Discussion of current therapeutic strategies.

Main Results:

  • Identification of prevalent APAH-CHD subtypes.
  • Framework for interpreting hemodynamic profiles.
  • Stratification of patients based on management needs.

Conclusions:

  • Accurate diagnosis and classification of APAH-CHD are crucial for effective treatment.
  • Hemodynamic assessment is key to understanding disease severity and guiding therapy.
  • Targeted medical treatments are evolving for APAH-CHD patients.

Keywords:
Congenital heart diseaseEisenmenger syndromeFontanOperabilityPulmonary arterial hypertension

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